Written for pulmonary clinicians and scientists alike, Molecular Basis of Lung Disease: Insights from Rare Disorders is a comprehensive and invaluable nesource that sheds new light on the molecular mechanisms influencing the clinical presentation and [books.google.de]

(OI) PowerPoint Presentation: A-aDO2 PAO2-Pao2 Normal range in a new born :5-15 Abnormal :15-40 Definitely abnormal: 40 PowerPoint Presentation: a/A Ratio Ratio of Pao2 to PAO2 It is a better indicator of gas exchange as the ratio is less affected by [authorstream.com]

We present a patient with a novel point mutation resulting in hereditary Surfactant Protein-B (SP-B) deficiency. [content.iospress.com]

Abstract We report on a term newborn with an unusual presentation and course of a rare lung disease due to mutation in SFTPC gene. [nature.com]

SP-B mRNA was present in all lung samples except that of the case patient, in which no signal was detected even after prolonged exposure. [nejm.org]

• Respiratory Distress

  Respiratory Distress Syndrome, Infant Synonyms of Respiratory Distress Syndrome, Infant Hyaline Membrane Disease Infantile Respiratory Distress Syndrome IRDS Subdivisions of Respiratory Distress Syndrome, Infant surfactant protein-B deficiency General [rarediseases.org]

  APPROACH TO RESPIRATORY DISTRESS IN NEWBORN: APPROACH TO RESPIRATORY DISTRESS IN NEWBORN BY DR JAYA PRAKASH PowerPoint Presentation: Definition Presence of at least 2 of the fallowing features are essential 1)Tachypnea (RR 60 PER MIN..) 2)Retractions [authorstream.com]

  A synthetic surfactant is commercially available for use in the treatment of respiratory distress syndrome of the newborn. See also acute respiratory distress syndrome. sur·fac·tant ( sŭr-fak'tănt ), 1. [medical-dictionary.thefreedictionary.com]

  Infant respiratory distress syndrome ( IRDS ), also called neonatal respiratory distress syndrome ( NRDS ), [1] respiratory distress syndrome of newborn, or increasingly surfactant deficiency disorder ( SDD ), [2] and previously called hyaline membrane [en.wikipedia.org]

  Title: [Relationship between R236C site in exon 7 of SP-B gene and respiratory distress syndrome in Han newborns in western Inner Mongolia]. Rare mutations in surfactant-associated genes contribute to neonatal respiratory distress syndrome. [ncbi.nlm.nih.gov]

• Dyspnea

  The natural course of untreated RDS is characterized by progressive worsening of cyanosis and dyspnea. [clinicalgate.com]

  Most of these cases were less severely affected, had dyspnea and progressive cough, sometimes accompanied by cyanosis, finger clubbing, failure to thrive in the younger ones, and asthenia or weight loss in the others. [respiratory-research.biomedcentral.com]

• Pulmonary Disorder

  These data support the feasibility of using immunoanalyses of BAL fluid to evaluate chronic pediatric pulmonary disorders in more detail. [respiratory-research.biomedcentral.com]

  SP-B was readily detected in the lung tissue of the control patients with various pulmonary disorders but was absent in the case patient's lung tissue ( Figure 1 ). SP-A was readily detected in all lung samples in roughly comparable amounts. [nejm.org]

• Sputum

  SP-D Which of the following proteins is found to be deficient in the sputum of patients with asthma? SP-A Which of the following statements refer to the Bear Cub 750vs infant ventilator? I. In A/C mode, mandatory breaths r time or flow triggered. [studystack.com]

• Suggestibility

  Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models [books.google.de]

  Anne Murphy and Arnold Strauss for helpful suggestions. [nejm.org]

  We suggest heightened vigilance and consultation with a reference laboratory in detecting similar mutations in term newborns presenting with unexplained respiratory failure. [content.iospress.com]

  These results suggest that SP-B and SP-C have different contributions to inter- and intra-membrane lipid dynamics, and that their combined action could provide unique effects to modulate structure and dynamics of pulmonary surfactant membranes and films [biochemj.org]

  (PMID: 16456143) Hersh CP … Silverman EK (American journal of respiratory and critical care medicine 2006) 3 22 45 60 Family-based association tests suggest linkage between surfactant protein B (SP-B) (and flanking region) and respiratory distress syndrome [genecards.org]

[…] suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models, clinical presentation, diagnostic approaches, conventional management and treatment [books.google.de]

Standard Therapies Treatment of infant respiratory distress syndrome consists of mechanical or physical breathing assistance such as positive end expiratory pressure (PEEP). Other treatment is symptomatic and supportive. [rarediseases.org]

which became a more established treatment modality. [healio.com]

Intratracheal surfactant Supplementary oxygen as needed Mechanical ventilation as needed Specific treatment of RDS is intratracheal surfactant therapy. [msdmanuals.com]

Pharmacologic Therapies Several pharmacologic options are available to the clinician for the treatment of RDS and prevention of its complications. Selected treatments are reviewed here. [clinicalgate.com]

Prognosis with treatment is excellent; mortality is 10%. With adequate ventilatory support alone, surfactant production eventually begins, and once production begins, RDS resolves within 4 or 5 days. [msdmanuals.com]

Finally, the prognosis for infants with congenital alveolar proteinosis has been uniformly poor, even with the use of extracorporeal membrane oxygenation. Therapeutic whole-lung lavage has also been considered 4. [nejm.org]

Transient tachypnea Pneumonia Aspiration syndromes Pneumothorax Pulmonary hypoplasia Pleural effusions Airway problems Hyaline membrane disease Pneumonia Pulmonary hemorrhage Air leak syndromes Pulmonary hypoplasia Bronchopulmonary dysplasia Laryngomalacia Etiology [authorstream.com]

The etiology of BPD is problematic and may be due to oxygen, overventilation or underventilation. [en.wikipedia.org]

It is occasionally difficult to distinguish respiratory from nonrespiratory etiologies on the basis of clinical signs alone. [clinicalgate.com]

Thus, despite similar chest x-rays and histopathological findings, the BAL profile for SP-B, SP-C and their proforms appears useful in distinguishing PAP from SP-B deficiency of any etiology. [respiratory-research.biomedcentral.com]

Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models [books.google.de]

Relevant External Links for SFTPB Genetic Association Database (GAD) SFTPB Human Genome Epidemiology (HuGE) Navigator SFTPB Atlas of Genetics and Cytogenetics in Oncology and Haematology: SFTPB No data available for Genatlas for SFTPB Gene Polymorphic [genecards.org]

HMD - Pathophysiology: HMD - Pathophysiology Terminal airspaces are not uniform throughout the lung There is a range of critical opening and critical closing pressures in various alveoli Lung Function in HMD: Lung Function in HMD Lung Compliance is also [authorstream.com]

This chapter describes the pathophysiology and molecular biology of hereditary SP-B deficiency. [ommbid.mhmedical.com]

Which of the following pathophysiologic conditions are components of meconium aspiration? I. Surfactant inactivation II. [studystack.com]

Pathophysiology of respiratory distress syndrome. The above scenario is repeated nightly throughout the United States with private pediatricians caring for such infants on a regular basis. [healio.com]

Pathophysiology [ edit ] The lungs of infants with respiratory distress syndrome are developmentally deficient in a material called surfactant, which helps prevent collapse of the terminal air-spaces (the future site of alveolar development) throughout [en.wikipedia.org]

Extensive testing of each preparation has demonstrated that both products are efficacious in preventing and treating RDS and preventing the complications of pulmonary airleak. [healio.com]

The only way to prevent the need for surfactant replacement therapy is to prevent a premature birth. [medical-dictionary.thefreedictionary.com]

Timely and appropriate therapy is essential to prevent ongoing injury and improve outcome. [clinicalgate.com]

Figure 5 The airspaces are lined with a lipoprotein complex called pulmonary surfactant, named for its ability to maintain normal respiratory mechanics by reducing surface tension at the air/liquid interface, preventing the lungs from collapsing at the [webs.ucm.es]

Prevention [ edit ] Giving the mother glucocorticoids speeds the production of surfactant. For very premature deliveries, a glucocorticoid is given without testing the fetal lung maturity. [en.wikipedia.org]